[Treatment of radial agenesis with the Ilizarov method]

Typical cases of lateral longitudinal hemimelia of the upper limb are characterized by agenesia of the radius and the thumb. The hand has four fingers and due to the lack of a radius, deviates laterally beyond 90 degrees. The ulna is short in 50 to 60% of the cases. Hand function is very limited causing a major handicap in everyday life activities. We report here the long-term outcome of 14 patients treated with the Ilizarov method.

Fourteen cases in thirteen patients, mean age 14.8 years, with radial hemimelia (one bilateral surgery) were treated with the Ilizarov method between June 1981 and December 1998 at the Lecco Hospital, Italy. Currently, the circular frame is composed of 3 elements: a half ring fixed by pins and wires to the metacarpal bones, 2 rings with pins and wires in the ulna, and 2 hinge clamps between the half-ring and the first distal ring to center the hand on the ulna by applying a lateral force. After centering the hand, the procedure continues with an ulnocarpal arthrodesis and is terminated by a proximal osteotomy of the ulna for forearm lengthening.

Patients were followed for 1 to 16 years. A last follow-up, hand function was excellent in 2 cases, good in 1, average in 10 and poor in 1. The esthetic result was excellent in 6, good in 6 and average in 2.

These results demonstrate that excellent stability of the hand centered on the ulna and forearm lengthening can be achieved with the new hybrid and more stable Ilizarov circular fixation method. Surgeons should become more familiar with this new instrumentation and its use.